Abstract
Mitochondria are double membrane organelles in eukaryotic cells that provide energy by generating adenosine triphosphate (ATP) through oxidative phosphorylation. They are crucial to many aspects of cellular metabolism. Mitochondria contain their own DNA that encodes for essential proteins involved in the execution of normal mitochondrial functions. Compared with nuclear DNA, the mitochondrial DNA (mtDNA) is more prone to be affected by DNA damaging agents, and accumulated DNA damages may cause mitochondrial dysfunction and drive the pathogenesis of a variety of human diseases, including neurodegenerative disorders and cancer. Therefore, understanding better how mtDNA damages are repaired will facilitate developing therapeutic strategies. In this review, we focus on our current understanding of the mtDNA repair system. We also discuss other mitochondrial events promoted by excessive DNA damages and inefficient DNA repair, such as mitochondrial fusion, fission, and mitophagy, which serve as quality control events for clearing damaged mtDNA.
Highlights
The mitochondria provide most of the cell energy in the form of adenosine triphosphate (ATP) through oxidative phosphorylation (OXPHOS), which is executed by the electron transport chain (ETC) within the mitochondria
We still lack a comprehensive understanding of how distinct pathways/mechanisms act together to maintain a functional mitochondrial genome
We have mainly focused on the mitochondrial response that repairs, attenuates, or eliminate mitochondrial DNA (mtDNA) damages
Summary
The mitochondria provide most of the cell energy in the form of adenosine triphosphate (ATP) through oxidative phosphorylation (OXPHOS), which is executed by the electron transport chain (ETC) within the mitochondria. We discuss a series of mitochondrial quality control events, including fission, fusion, and mitophagy, which are important for clearing damaged mtDNA that cannot be efficiently repaired. More and more studies have shown that several other DNA repair pathways, including base excision repair (BER), direct reversal (DR), mismatch repair (MMR), and possibly double-strand break repair (DSBR), exist in the mammalian mitochondria (Kazak et al, 2012; Saki and Prakash, 2017; Figure 1).
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